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2018 ; 13
(5
): e0197297
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Prevalence of primary Sjögren s syndrome in patients undergoing evaluation for
pulmonary arterial hypertension
#MMPMID29763424
Sato T
; Hatano M
; Iwasaki Y
; Maki H
; Saito A
; Minatsuki S
; Inaba T
; Amiya E
; Fujio K
; Watanabe M
; Yamamoto K
; Komuro I
PLoS One
2018[]; 13
(5
): e0197297
PMID29763424
show ga
BACKGROUND: The prevalence of pulmonary arterial hypertension (PAH) in primary
Sjögren's syndrome (SS) had been reported to be rare. However, recent studies
using echocardiography as a screening method showed conflicting results, and the
true prevalence is still unclear. Since diagnosing primary SS is difficult
because of its heterogeneous nature, a number of patients with
primary-SS-associated PAH may be misdiagnosed with idiopathic PAH, losing their
chance to undergo immunosuppressive therapy. Therefore, we sought to elucidate
the prevalence of primary SS among patients who initially present with PAH.
METHODS: From our prospective institutional PAH database, 40 consecutive patients
without any obvious cause of PAH at the time of PAH diagnosis were identified. We
retrospectively evaluated the prevalence of primary SS diagnosed during or after
the initial assessment of PAH. RESULTS: During the initial assessment, one
patient was diagnosed with primary-SS-associated PAH. Among the 25 patients who
were initially diagnosed with idiopathic PAH, five were diagnosed with primary SS
during their course of the disease. Of the five patients, three had key signs
suggesting primary SS and were probably underdiagnosed at the time of initial
evaluation. The remaining two patients, who were finally diagnosed with primary
SS, did not have any specific signs suggesting primary SS at the time of initial
evaluation but showed positive conversion of their autoantibodies during the
course of PAH. CONCLUSION: The prevalence of primary-SS-associated PAH may be
relatively high among patients who undergo initial evaluation for PAH.
Furthermore, primary-SS-associated PAH may be underdiagnosed with routine
evaluation for the primary cause of PAH. Clinicians should pay specific attention
and carefully evaluate the possibility of primary SS in patients with PAH.